title: Susac's syndrome: first from India and youngest in the world.
abstract: A two and half year old female was admitted at the emergency room suffering from gradually worsening headache followed by nausea. The child presented with reduced level of consciousness and bilateral hypoacusis. The patient was lethargic. Ophthalmic examination showed branch retinal artery occlusion (BRAO). This finding was crucial to the diagnosis of Susac's syndrome (SS), a rare autoimmune disease characterized by, endotheliopathy of retina, encephalic tissues and cochlea. Magnetic resonance imaging of the brain also showed typical features. Thorough blood investigations did not reveal any other abnormality. Patient was treated with immunosuppressive to prevent her from developing severe sequelae of this disease. The child showed dramatic improvement in her systemic condition within 48 h of starting the treatment. This is the youngest ever and first case report from India.
authors.affiliation: Department of Ophthalmology, Sarojini Naidu Medical College, Agra, India.
authors.forename: Gunjan
authors.initials: G
authors.lastname: Prakash
authors.forename: Shephali
authors.initials: S
authors.lastname: Jain
authors.forename: Mausam
authors.initials: M
authors.lastname: Gupta
authors.forename: Tirupat
authors.initials: T
authors.lastname: Nathi
mesh_terms: D001921:Brain / Q000473:pathology*; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007194:India; D008279:Magnetic Resonance Imaging / Q000379:methods; D012160:Retina / Q000473:pathology*; D055955:Susac Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D041623:Tomography, Optical Coherence
chemical_list: D005938:Glucocorticoids